ICD-10-CM 2027
ICD-10 code for Maroteaux-Lamy syndrome
From the official ICD-10-CM alphabetic index entry “Maroteaux-Lamy syndrome (mild) (severe)”. Page updated September 29, 2026.
About coding Maroteaux-Lamy syndrome
The ICD-10-CM code for Maroteaux-Lamy syndrome is E76.29 (Other mucopolysaccharidoses).
Within E76.2, choose E76.29 (Other mucopolysaccharidoses) only when documentation doesn't support a more specific option: Morquio mucopolysaccharidoses (E76.21) and Sanfilippo mucopolysaccharidoses (E76.22).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Maroteaux-Lamy syndrome ICD-10 codes
What is the ICD-10 code for Maroteaux-Lamy syndrome?
- E76.29 — Other mucopolysaccharidoses.
Is E76.29 billable?
- Yes. E76.29 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E76.29 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E76.29 as a principal diagnosis.
Is E76.29 a CC or MCC?
- E76.29 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.